scholarly journals Management of NK/T-Cell Lymphoma, Nasal Type

2019 ◽  
Vol 15 (10) ◽  
pp. 513-520 ◽  
Author(s):  
Pamela B. Allen ◽  
Mary Jo Lechowicz

Extranodal natural killer (NK)/T-cell lymphoma, nasal type, is a rare peripheral T-cell lymphoma associated with Epstein-Barr virus. It most often presents as limited-stage disease in patients of East Asian descent with a palatal deformity caused by erosion of the tumor through the hard palate. Limited-stage disease is often curable with the use of l-asparaginase–based chemotherapy and high-dose radiation therapy. Obtaining an accurate diagnosis is essential, because treatment with standard lymphoma regimens and omission of radiation severely compromise the likelihood of long-term survival. Conversely, patients with advanced disease have a poor prognosis and are recommended for asparaginase-based chemotherapy followed by consolidation with autologous transplantation as a potentially curative approach. Progress often has been hampered by the rarity of this disease. However, discovery of common genetic alterations in pathways that promote growth and inhibit apoptosis, and actionable markers such as CD30 (among others), have begun to broaden the availability of novel drugs (eg, targeted therapies). There is also cautious optimism about immunotherapies, such as checkpoint blockade and novel cellular therapies that target Epstein-Barr virus. Advances in treatment and understanding of the genetic landscape of this disease offer hope for improved treatment outcomes.

2017 ◽  
Vol 56 (11) ◽  
pp. 1409-1414 ◽  
Author(s):  
Ayano Imai ◽  
Hiroshi Takase ◽  
Ken-Ichi Imadome ◽  
Go Matsuda ◽  
Iichiro Ohnishi ◽  
...  

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 5363-5363
Author(s):  
Chengcheng Guo ◽  
He Huang ◽  
Ying Tian ◽  
Xueying Li ◽  
Mengping Zhang ◽  
...  

Abstract Predictive value of therapeutic monitoring with plasma Epstein-Barr virus DNA in Extranodal NK/T cell lymphoma, nasal type Background: Predictive tumor markers are essential for extranodal NK/T cell lymphoma, nasal type (ENKTL), which pursues an aggressive clinical course with poorer prognosis. This prospective study was conducted to evaluate the dynamic monitoring value of circulating EBV DNA concentration for the prediction of ENKL during treatment. Method: Patients with untreated, centrally reviewed diagnosis of ENKTL were included in our study. Plasma Epstein-Barr virus (EBV) DNA levels were collected before and/or every 2 cycles of chemotherapies for circulating EBV DNA measurement by a real-time PCR assay. Result: From Jan 2002 to Aug 2012, 113 newly diagnostic ENKTL patients enrolled. The positive rate of circulating EBV DNA is 61.9% (70/113) with a median concentration of 1.21×103copies/ml. Patients who had initial positive circulating EBV-DNA had more lymph nodes invasion, higher IPI and KPI score. The more advance of the stage, the higher rate of the circulating EBV-DNA positive. When divided the positive group as low and high-dose ones by the cut-off value as 2×104copies/ml, the CR rate of the high-dose group is much lower and the 5-year OS is significantly better than the low-dose group and the negative group. After two cycles of chemotherapy, 45 patients were tested circulating EBV-DNA and 53.33% (24/45) patients became negative EBV-DNA candidates. There were 87.5% (21/24) patients obtained complete remission at the end of the treatment, comparing as 42.86% (9/21) in the still positive EBV-DNA groups(P= 0.002). There is a tendency that the patients whose circulating EBV-DNA become negative after two cycles will have better prognosis (5-year OS: 75% vs 46%, P=0.074). The similar situation of CR rate and OS happened in the EBV-DNA detection of completion of total chemotherapy. 7 of 13 relapsed pts of >1×103copies/ml EBV-DNA at the time of recurrent, and the survival outcome was dismal for them compared to the other 6 pts of ≤1×103copies/ml(5- year OS: 0% vs 80%, P=0.001) Conclusion: Circulating EBV-DNA level can predict the efficacy of treatment as a dynamic marker of ENKL. Patients with early positive detection of EBV-DNA after 2 cycles of chemotherapy maybe received more aggressive treatments. Disclosures No relevant conflicts of interest to declare.


2018 ◽  
Vol 23 (2) ◽  
pp. 1509-1516
Author(s):  
Weili Xue ◽  
Weiming Li ◽  
Yufeng Shang ◽  
Yanjie Zhang ◽  
Xuan Lan ◽  
...  

2021 ◽  
Vol 14 (11) ◽  
pp. e235100
Author(s):  
Adora Tricia Santos ◽  
Jiankun Tong ◽  
Amir Steinberg ◽  
Larry Shemen

Infection with Epstein-Barr virus (EBV) has been linked to approximately 10%–15% of lymphomas diagnosed in the USA, including a small percentage of Natural Killer (NK)/T cell lymphomas, which are clinically aggressive, respond poorly to chemotherapy and have a shorter survival. Here, we present a case of a patient found to have EBV-induced NK/T cell lymphoma from a chronic EBV infection. While the EBV most commonly infects B cells, it can infect NK/T cells, and it is important for the clinician to be aware of the potential transformation to lymphoma as it is clinically aggressive, warranting early recognition and treatment. NK/T cell lymphoma is a unique type of non-Hodgkin's lymphoma that is almost always associated with EBV. The disease predominantly localises in the upper aerodigestive tract, most commonly in the nose.


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